Huge peripheral primitive neuroectodermal tumor of the small bowel mesentery at nonage: A case report and review of the literature

Abstract

Extraskeletal Ewing's sarcoma/peripheral primitive neuroectodermal tumor (E-EWS/pPNET) is a rare aggressive malignant small round cell tumor. In this report, we present the case of a 15-year-old boy who suffered from acute abdominal pain accompanied by hematemesis and melena, and was eventually diagnosed with E-EWS/pPNET. To date, there have been only five reported cases of E-EWS/pPNET of the small bowel including the patient in this report. To the best of our knowledge, this is the first documentation of a pPNET of the small bowel mesentery at nonage. All these have made this report rare and significant.

Keywords: Extraskeletal Ewing’s sarcoma; Nonage; Peripheral primitive neuroectodermal tumor; Small bowel mesentery; Spontaneous rupture.

Figure 1

Computerized tomography scan revealing a large ovoid solid and cystic tumor (17 cm × 15 cm × 10 cm) at the left upper quadrant (right arrow).

Figure 2

Macroscopically, a large ovoid solid and cystic tumor (20 cm × 20 cm × 10 cm) was observed at the left upper quadrant, and the tumor infiltrated the full-thickness wall of the jejunum (left arrow).

Figure 3

Macroscopically, an incision into the surface of the tumor revealed sclerotic tissue (up arrow) and bleeding regions inside the tumor (left arrow). The tumor had infiltrated the full-thickness wall of the jejunum (right arrow).

Figure 4

Small round cells containing uniform vesicular and Homer-Wright rosettes were found microscopically (right arrow, × 200).

Figure 5

Red blood cells were found between the tumor cells (right arrow, × 200).